PMID- 21303426 OWN - NLM STAT- MEDLINE DCOM- 20110831 LR - 20221207 IS - 1440-1843 (Electronic) IS - 1323-7799 (Linking) VI - 16 IP - 4 DP - 2011 May TI - Genetic predisposition to diffuse panbronchiolitis. PG - 581-8 LID - 10.1111/j.1440-1843.2011.01946.x [doi] AB - Diffuse panbronchiolitis is characterized by chronic inflammation in respiratory bronchioles and sinobronchial infection. The pathophysiology accompanying the persistent bacterial infection is noteworthy for the accumulation of lymphocytes and foamy macrophages around the small airways, for mucus hypersecretion, and for the number of neutrophils in the large airways. Until the establishment of long-term macrolide therapy, the prognosis was generally poor. Case studies of diffuse panbronchiolitis in East Asians, including Japanese, Koreans and Chinese, have frequently been reported, and genetic predisposition to the disease has been assumed in Asians. Immunogenetic studies revealed a strong association with human leukocyte antigen (HLA)-B54 in Japanese, whereas an association with HLA-A11 was reported in Koreans. These findings imply that a major susceptibility gene may be located between the HLA-A and HLA-B loci on the short arm of human chromosome 6. We have recently cloned novel mucin-like genes in this candidate region. In addition to accumulated knowledge of classical HLA genes and mucin genes, further analysis of newly identified genes may provide insights into the pathogenesis of the disease. CI - (c) 2011 The Authors. Respirology (c) 2011 Asian Pacific Society of Respirology. FAU - Keicho, Naoto AU - Keicho N AD - Department of Respiratory Diseases, Research Institute, National Center for Global Health and Medicine, Tokyo, Japan. nkeicho-tky@umin.ac.jp FAU - Hijikata, Minako AU - Hijikata M LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - Australia TA - Respirology JT - Respirology (Carlton, Vic.) JID - 9616368 RN - 0 (HLA Antigens) RN - 0 (Macrolides) RN - 0 (Mucins) RN - Diffuse panbronchiolitis SB - IM MH - *Bronchiolitis/drug therapy/genetics/immunology MH - Chromosomes, Human, Pair 6/genetics MH - Cystic Fibrosis/genetics/immunology MH - Asia, Eastern MH - Female MH - Foam Cells/drug effects/immunology MH - *Genetic Predisposition to Disease MH - HLA Antigens/genetics/immunology MH - *Haemophilus Infections/drug therapy/genetics/immunology MH - Humans MH - Lymphocytes/drug effects/immunology MH - Macrolides/therapeutic use MH - Male MH - Mucins/genetics/immunology MH - Mucus/drug effects/metabolism MH - Neutrophils/drug effects/immunology MH - Pacific Islands MH - Polymorphism, Genetic EDAT- 2011/02/10 06:00 MHDA- 2011/09/01 06:00 CRDT- 2011/02/10 06:00 PHST- 2011/02/10 06:00 [entrez] PHST- 2011/02/10 06:00 [pubmed] PHST- 2011/09/01 06:00 [medline] AID - 10.1111/j.1440-1843.2011.01946.x [doi] PST - ppublish SO - Respirology. 2011 May;16(4):581-8. doi: 10.1111/j.1440-1843.2011.01946.x.